Mesenteric fibromatosis is a proliferative fibroblastic neoplasia of the small intestine mesentery which may occur as a unique or multiple formation. Mesenteric fibromatosis represents the 8% of all desmoid neoplasm. Giant mesenteric fibromatosis is uncommon by itself (2-4 case/milion/year). Since the rarity of this tumor and the difficulties in diagnostic and therapeutic ambit, we believe it justified to describe a case of giant mesenteric fibromatosis which came to our observation.